Porphyria, Variegate


Deficiencies, Protoporphyrinogen Oxidase

Porphyria Variegata

Porphyria Variegate

Porphyria Variegates

Ppox Deficiency

Protoporphyrinogen Oxidase Deficiency

Variegate Porphyria

Variegate, Porphyria

Variegates, Porphyria

An autosomal dominant porphyria that is due to a deficiency of protoporphyrinogen oxidase (EC in the LIVER, the seventh enzyme in the 8-enzyme biosynthetic pathway of HEME. Clinical features include both neurological symptoms and cutaneous lesions. Patients excrete increased levels of porphyrin precursors, COPROPORPHYRINS and protoporphyrinogen.